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Understanding How Cutaneous Lupus Erythematosus Progresses to Systemic Lupus Erythematosus

Understanding How Cutaneous Lupus Erythematosus Progresses to Systemic Lupus Erythematosus Epidemiological studies have reported that up to 23% of patients with cutaneous lupus erythematosus (CLE) develop systemic lupus erythematosus (SLE), with some progressing over several years.1 As these data have been important in estimating one’s risk of systemic spread, how CLE progresses to SLE in these patients is unknown. This article by Wieczorek et al2 seeks to address this practice gap by evaluating the severity of symptoms and SLE criteria that are seen and met in patients with CLE who develop SLE. Wieczorek et al2 prospectively follow 77 patients with CLE and determine that 17% eventually met criteria for SLE diagnosis. These 13 patients mostly fulfilled mucocutaneous criteria, and a minority of these (38%) displayed new moderate to severe systemic disease. These findings underscore that CLE progresses to SLE in a significant minority of patients and suggest that most patients with CLE who progress to SLE do not experience the severe manifestations of lupus, which have multiple clinical implications. First, it uncovers a practice gap because this insidious progression can be easily overlooked. Health care clinicians need to remain vigilant for concerning systemic symptoms and signs by conducting a complete review of systems (eg, joint swelling/pain in the small joints) at each clinic visit and ordering periodic laboratory tests such as complete blood cell counts with differentials and urinalyses at least once annually. A positive review of systems would prompt additional serologic tests (eg, antinuclear antibodies, anti–double-stranded DNA, anti-Smith antibodies), but they are not recommended in the absence of systemic symptoms. Second, a new SLE diagnosis in CLE may not require a specialist referral if they have skin-predominant disease. More than 30% of the patients with CLE that progressed to SLE in this study met criteria related to cutaneous findings with and without autoantibody abnormalities. Finally, we can educate these patients with this information to assuage concerns about their disease prognosis. Compared with an earlier epidemiological study,1 Wieczorek et al2 showed similar data showing that the mean duration between CLE and SLE diagnosis was 8.02 years. While this implies a gradual disease progression in these patients, this could also be due to delays in SLE diagnosis. Closing this practice gap would require improvements in educating health care clinicians on developing focused review of systems and evaluating laboratory test results. Widespread acceptance of this practice can occur with dermatologists embracing the responsibility of screening for SLE in patients with CLE. The article also identifies specific risk factors for progression such as positive antinuclear antibodies, widespread discoid lesions, female sex, and increased numbers of SLE criteria. While these particular characteristics will heighten the chances for systemic progression, the profile of these high-risk patients is not fully characterized. The Cutaneous Lupus Registry at University of Texas Southwestern Medical Center is prospectively studying patients with CLE who do and do not develop SLE to determine both clinical features and biomarkers that generate a thorough characterization of these high-risk patients. This form of “personalized” medicine can revolutionize treatment of patients with CLE because those at high risk for progression to SLE could be promptly started on antimalarial therapy, which can delay the onset of SLE.3 Back to top Article Information Corresponding Author: Benjamin F. Chong, MD, Department of Dermatology, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX 75309-9069 (ben.chong@utsouthwestern.edu). Published Online: January 29, 2014. doi:10.1001/jamadermatol.2013.9030. Conflict of Interest Disclosures: Dr Chong is an investigator for Daavlin Corporation. References 1. Durosaro O, Davis MD, Reed KB, Rohlinger AL. Incidence of cutaneous lupus erythematosus, 1965-2005: a population-based study. Arch Dermatol. 2009;145(3):249-253.PubMedGoogle ScholarCrossref 2. Wieczorek IT, Propert KJ, Okawa J, Werth VP. Systemic symptoms in the progression of cutaneous to systemic lupus erythematosus [published online January 29, 2014]. JAMA Dermatol. doi:10.1001/jamadermatol.2013.9026.Google Scholar 3. James JA, Kim-Howard XR, Bruner BF, et al. Hydroxychloroquine sulfate treatment is associated with later onset of systemic lupus erythematosus. Lupus. 2007;16(6):401-409.PubMedGoogle ScholarCrossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png JAMA Dermatology American Medical Association

Understanding How Cutaneous Lupus Erythematosus Progresses to Systemic Lupus Erythematosus

JAMA Dermatology , Volume 150 (3) – Mar 1, 2014

Understanding How Cutaneous Lupus Erythematosus Progresses to Systemic Lupus Erythematosus

Abstract

Epidemiological studies have reported that up to 23% of patients with cutaneous lupus erythematosus (CLE) develop systemic lupus erythematosus (SLE), with some progressing over several years.1 As these data have been important in estimating one’s risk of systemic spread, how CLE progresses to SLE in these patients is unknown. This article by Wieczorek et al2 seeks to address this practice gap by evaluating the severity of symptoms and SLE criteria that are seen and met in patients with...
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Publisher
American Medical Association
Copyright
Copyright © 2014 American Medical Association. All Rights Reserved.
ISSN
2168-6068
eISSN
2168-6084
DOI
10.1001/jamadermatol.2013.9030
pmid
24477964
Publisher site
See Article on Publisher Site

Abstract

Epidemiological studies have reported that up to 23% of patients with cutaneous lupus erythematosus (CLE) develop systemic lupus erythematosus (SLE), with some progressing over several years.1 As these data have been important in estimating one’s risk of systemic spread, how CLE progresses to SLE in these patients is unknown. This article by Wieczorek et al2 seeks to address this practice gap by evaluating the severity of symptoms and SLE criteria that are seen and met in patients with CLE who develop SLE. Wieczorek et al2 prospectively follow 77 patients with CLE and determine that 17% eventually met criteria for SLE diagnosis. These 13 patients mostly fulfilled mucocutaneous criteria, and a minority of these (38%) displayed new moderate to severe systemic disease. These findings underscore that CLE progresses to SLE in a significant minority of patients and suggest that most patients with CLE who progress to SLE do not experience the severe manifestations of lupus, which have multiple clinical implications. First, it uncovers a practice gap because this insidious progression can be easily overlooked. Health care clinicians need to remain vigilant for concerning systemic symptoms and signs by conducting a complete review of systems (eg, joint swelling/pain in the small joints) at each clinic visit and ordering periodic laboratory tests such as complete blood cell counts with differentials and urinalyses at least once annually. A positive review of systems would prompt additional serologic tests (eg, antinuclear antibodies, anti–double-stranded DNA, anti-Smith antibodies), but they are not recommended in the absence of systemic symptoms. Second, a new SLE diagnosis in CLE may not require a specialist referral if they have skin-predominant disease. More than 30% of the patients with CLE that progressed to SLE in this study met criteria related to cutaneous findings with and without autoantibody abnormalities. Finally, we can educate these patients with this information to assuage concerns about their disease prognosis. Compared with an earlier epidemiological study,1 Wieczorek et al2 showed similar data showing that the mean duration between CLE and SLE diagnosis was 8.02 years. While this implies a gradual disease progression in these patients, this could also be due to delays in SLE diagnosis. Closing this practice gap would require improvements in educating health care clinicians on developing focused review of systems and evaluating laboratory test results. Widespread acceptance of this practice can occur with dermatologists embracing the responsibility of screening for SLE in patients with CLE. The article also identifies specific risk factors for progression such as positive antinuclear antibodies, widespread discoid lesions, female sex, and increased numbers of SLE criteria. While these particular characteristics will heighten the chances for systemic progression, the profile of these high-risk patients is not fully characterized. The Cutaneous Lupus Registry at University of Texas Southwestern Medical Center is prospectively studying patients with CLE who do and do not develop SLE to determine both clinical features and biomarkers that generate a thorough characterization of these high-risk patients. This form of “personalized” medicine can revolutionize treatment of patients with CLE because those at high risk for progression to SLE could be promptly started on antimalarial therapy, which can delay the onset of SLE.3 Back to top Article Information Corresponding Author: Benjamin F. Chong, MD, Department of Dermatology, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX 75309-9069 (ben.chong@utsouthwestern.edu). Published Online: January 29, 2014. doi:10.1001/jamadermatol.2013.9030. Conflict of Interest Disclosures: Dr Chong is an investigator for Daavlin Corporation. References 1. Durosaro O, Davis MD, Reed KB, Rohlinger AL. Incidence of cutaneous lupus erythematosus, 1965-2005: a population-based study. Arch Dermatol. 2009;145(3):249-253.PubMedGoogle ScholarCrossref 2. Wieczorek IT, Propert KJ, Okawa J, Werth VP. Systemic symptoms in the progression of cutaneous to systemic lupus erythematosus [published online January 29, 2014]. JAMA Dermatol. doi:10.1001/jamadermatol.2013.9026.Google Scholar 3. James JA, Kim-Howard XR, Bruner BF, et al. Hydroxychloroquine sulfate treatment is associated with later onset of systemic lupus erythematosus. Lupus. 2007;16(6):401-409.PubMedGoogle ScholarCrossref

Journal

JAMA DermatologyAmerican Medical Association

Published: Mar 1, 2014

References